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Susac Syndrome

What is Susac Syndrome?

Susac syndrome is an autoimmune endotheliopathy, meaning the body’s immune system mistakenly attacks the endothelium. This is the thin layer of cells that lines the inner walls of blood vessels,

Susac syndrome typically affects the smallest blood vessels in the brain, retina, and inner ear. When endothelial cells are attacked and injured, they swell and obstruct blood flow within the vessels. The blockage reduces the amount of oxygen and nutrients delivered to the brain, retina, and inner ear, resulting in temporary dysfunction or permanent damage of these organs.

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Symptoms of Susac Syndrome

Symptoms involving the brain, retina, and inner ear may not appear at the same time. In some cases, it can take weeks, months, or even years to have symptoms in all three areas. 

Brain symptoms may include:

  • Cognitive problems, such as short-term memory loss, confusion, and difficulty problem-solving
  • Personality changes
  • Psychiatric symptoms
  • Severe migraine-like headaches, often with vomiting
  • Slurred speech

Eye symptoms may include:

  • Loss of peripheral vision
  • Visual disturbance, such as a dark spot in the field of vision in one or both eyes

Inner ear symptoms may include:

  • Dizziness (vertigo)
  • Hearing loss, especially at low frequencies, in one or both ears
  • Ringing in the ears (tinnitus)

Susac syndrome shares symptoms with other conditions. If you are concerned about your health, please contact a medical professional for an accurate diagnosis.

doctor consoling senior patient

Treatments for Susac Syndrome

Susac syndrome can be treated with immunosuppressant drugs. These include corticosteroids like prednisone and intravenous immunoglobulin (IVIg). Early, aggressive, and long-term corticosteroid treatment can prevent or minimize irreversible damage to the organs affected. The dosage may be able to be decreased over time. In some cases, Susac syndrome will spontaneously subside.

Additional Information

How common is Susac syndrome?

Susac syndrome is considered rare, but the National Organization of Rare Disorders says the true frequency is likely unknown due to misdiagnosis. Symptoms of Susac syndrome can mimic those of other inflammatory diseases, such as multiple sclerosis, acute disseminated encephalomyelitis, encephalitis, and vasculitis.

Who gets Susac syndrome?

Susac syndrome is most common in women between the ages of 20 and 40. It is unknown what causes the immune system to mistakenly attack the endothelium.

How is Susac syndrome diagnosed?

Susac syndrome is easier to diagnose when the brain, retina, and inner ear are all affected. Sometimes, however, people only have symptoms in one of the three areas at the time they seek a diagnosis.

The following may be used to diagnose Susac syndrome:

  • Audiogram (hearing test)
  • Fluorescein angiography to look at blood flow in the eye
  • Medical history
  • Physical examination
  • Neurological examination
  • Magnetic resonance imaging (MRI)

 Additional Information & Resources:

National Organization of Rare Disorders

Group 12
  Years
Susac syndrome is most common in women between the ages of 20 and 40.